Abstract
Abstract A 7-year-old girl with a six-month history of increasing virilism, growth, and acne excreted supranormal amounts of urinary 17-ketosteroids (87.6 mg/24 h). ACTH stimulation and dexamethasone suppression studies indicated adrenal tumor. A large left adrenal carcinoma was removed. Metastases were not observed. Postoperatively, 24-h urinary ketosteroids remained supranormal (16 mg). Sudden fever, cough, and hemoptysis precipitated hospitalization 42 months later. Bilateral lung metastases, a mass in the right upper quadrant, virilism, advanced bone age, and supranormal 24-h urinary ketosteroids (166 mg) and hydroxysteroids (16 mg) were found. The patient received 240 g of o,p'-dichlorodiphenyl-dichloroethane (o,p'-DDD), whereupon the abdominal mass and lung lesions regressed and 24-h urinary ketosteroids and hydroxysteroids decreased. Since this treatment (which ended April, 1968) the patient has been maintained at home on 7.5 g o,p'-DDD/day and replacement therapy with 9-α-fluorohydrocortisone and cortisone acetate. Almost all signs and symptoms have disappeared and urinary steroids are normal.
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